ACR Meeting Abstracts

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  • Abstract Number: 1237 • 2014 ACR/ARHP Annual Meeting

    Retroperitoneal Fibrosis and IgG4 Disease: Response to Immunosuppressive Therapy – a Single Centre Retrospective Study

    Shirish Sangle1, Pamela Lutalo2, Louise Nel3, James Pattison4, Tim OBrien5 and David P. D'Cruz6, 1Rheumatology, Louise Coote Lupus Unit, Guy's and St Thomas' Hospital, London, United Kingdom, 2Peter Gorer Department of Immunobiology, King's College London School of Medicine, London, United Kingdom, 3Rheumatology, Guy's and St Thomas' Hospital NHS Foundation Trust, London, United Kingdom, 4Nephrology, Guy's and St Thomas' Hospital NHS Foundation Trust, London, United Kingdom, 5Urology, Guy's and St Thomas' Hospital NHS Trust, London, United Kingdom, 6Louise Coote Lupus Unit, Guy's and St Thomas' Hospital, London, United Kingdom

    Background/Purpose: A retrospective observational study of patients with IgG4 related disease and retroperitoneal fibrosis Methods: Retroperitoneal fibrosis (RPF) is a rare chronic inflammatory condition which…
  • Abstract Number: 1236 • 2014 ACR/ARHP Annual Meeting

    IgG4 Immunostaining Is Common but Not Specific in Orbitbal Inflammatory Diseases

    James T. Rosenbaum1,2, Amanda Wong3, Patrick Stauffer3, Megan Troxell4, Donald Houghton5, Dongseok Choi6, Christine Harrington7, David Wilson8, Hans Grossniklaus9, Roger Dailey3, John Ng3, Eric Steele3, Patrick Yeatts10, Peter Dolman11, Valerie White11, Craig Czyz12, Jill Foster12, Deepak Edward13, Hind Alkatan13, Don Kikkawa14, Bobby Korn15, Dinesh Selva16, Gerald Harris17, Michael Kazim18, Payal Patel18 and Stephen R. Planck19, 1OHSU, Portland, OR, 2Arthritis and Rheumatic diseases, Oregon Health and Science University, Portland, OR, 3Oregon Health & Science University, Portland, OR, 4Pathology, Oregon Health and Science University, Portland, OR, 5Pathology, Oregon Health & Science University, Portland, OR, 6Casey Eye Institute, Oregon Health and Science University, Portland, OR, 7Integrated Genomics, Oregon Health & Science University, Portland, OR, 8Casey Eye Institute/Ophthalmology, Oregon Health & Science University, Portland, OR, 9Ophthalmology, Emory University, Atlanta, GA, 10Ophthalmology, Wake Forest University, Winston-Salem, NC, 11University of British Columbia, Vancouver, BC, Canada, 12Ohio State Univeristy, Columbus, OH, 13King Khaled Eye Hospital, Riyadh, Saudi Arabia, 14Ophthalmology, University of California, San Diego, San Diego, CA, 15University of California, San Diego, San Diego, CA, 16Royal Adelaide Hospital, Adelaide, Australia, 17Medical College of Wisconsin, Milwaukee, WI, 18Columbia University, New York City, NY, 19Casey Eye Inst/Ophthalmology, Oregon Health & Science University, Portland, OR

    Background/Purpose: IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we…
  • Abstract Number: 1235 • 2014 ACR/ARHP Annual Meeting

    Recovery of Renal Function after Corticosteroid Therapy for IgG4-Related Kidney Disease

    Takako Saeki1, Mitsuhiro Kawano2, Ichiro Mizushima2, Motohisa Yamamoto3, Yoshifumi Ubara4, Hitoshi Nakashima5, Yoko Wada6, Tomoyuki Ito7, Hajime Yamazaki7, Ichiei Narita6 and Takao Saito5,8, 1Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan, 2Kanazawa University Hospital, Kanazawa, Japan, 3Sapporo Medical University School of Medicine, Sapporo, Japan, 4Toranomon Hospital, Tokyo, Japan, 5Div of Nephrol & Rheumatol, Dept of Int Med, Faculty of Medicine, Fukuoka University, Fukuoka, Japan, 6Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan, 7Nagaoka Red Cross Hospital, Nagaoka, Japan, 8General Medical Research Center, Faculty of Medicine, Fukuoka University, Fukuoka, Japan

    Background/Purpose: In our earlier study of IgG4-related kidney disease (IgG4-RKD), we found that renal dysfunction, which was mostly attributable to IgG4-related tubulointerstitial nephritis, was significantly…
  • Abstract Number: 1234 • 2014 ACR/ARHP Annual Meeting

    Periodic Fever Syndromes in an Academic Medical Center

    Mark Cervinski1 and Daniel Albert2, 1Pathology, Dartmouth Hitchcock Medical Center, Lebanon, NH, 2Medicine, The Dartmouth Institute for Health Policy and Clinical Practice, lebanon, NH

    Background/Purpose: Most published clinical data on this rapidly evolving group of diseases  are from highly specialized centers and do not reflect what is commonly seen…
  • Abstract Number: 1233 • 2014 ACR/ARHP Annual Meeting

    Tocilizumab (TCZ) in the Treatment of AA Amyloidosis in Patients with Familial Mediterranean Fever

    Huri Ozdogan, Serdal Ugurlu, Aysa Hacioglu, Yasaman Adibnia and Vedat Hamuryudan, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: AA amyloidosis is the major long-term complication of various chronic inflammatory diseases like rheumatoid arthritis, ankylosing spondylitis, FMF and other autoinflammatory syndromes. Treatment of…
  • Abstract Number: 1232 • 2014 ACR/ARHP Annual Meeting

    Canakinumab Therapy in Patients with Familial Mediterranean Fever

    Serdal Ugurlu, Emire Seyahi, Gulen Hatemi, Aysa Hacioglu, Fatma Nihan Akkoc and Huri Ozdogan, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: According to a recent pilot study Canakinumab reduced the frequency of attacks in 9 patients with Familial Mediterranean Fever (FMF) resistant to colchicine with no…
  • Abstract Number: 1231 • 2014 ACR/ARHP Annual Meeting

    Evidence Based Recommendations for Genetic Diagnosis of Familial Mediterranean Fever

    Gabriella Giancane1, Nienke ter Haar2, Nico Wulffraat3, Bas Vastert4, Karyl Barron5, Veronique Hentgen6, Tilmann Kallinich7, Huri Ozdogan8, Jordi Anton9, Paul Brogan10, Luca Cantarini11, Joost Frenkel4, Caroline Galeotti12, Marco Gattorno13, Gilles Grateau14, Michael Hofer15, Isabelle Kone-Paut16, J B. Kuemmerle-Deschner17, Helen Lachmann18, Anna Simon19, Brian Feldman20, Yosef Uziel21 and Seza Ozen22, 1Pediatric Immunology, UMC, Utrecht, Netherlands, Utrecht, Netherlands, 2Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 3Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, 4University Medical Center Utrecht, Utrecht, Netherlands, 5NIH, Bethesda, MD, 6Versailles Hospital, Le Chesnay Cedex, France, 7Charite, University Medicine Berlin, Berlin, Germany, 8Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 9Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, 10Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 11University of Siena, Siena, Italy, 12Bicêtre Hospital, University of Paris SUD, Paris, France, 13Istituto Giannina Gaslini, Genova, Italy, 14Service De Médecine Interne, Hopital Tenon, Paris, France, 15Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, 16Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, 17Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 18UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, 19Radboudumc, Nijmegen, Netherlands, 20Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 21Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, 22Deptartment. of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey

    Background/Purpose: Familial Mediterranean Fever (FMF) is a disease that starts in childhood and can lead to significant morbidity. In 2013, an initiative called SHARE (Single…
  • Abstract Number: 1230 • 2014 ACR/ARHP Annual Meeting

    Efficacy of Interleukin-1 Targeting Drugs in Familial Mediterranean Fever Patients

    Pinar Cetin1, Ismail Sari1, Betul Sozeri2, Ozlem Cam2, Merih Birlik1, Fatos Onen1, Nurullah Akkoc1 and Servet Akar1, 1Rheumatology, Dokuz Eylul University School of Medicine, Izmir, Turkey, 2Pediatrics, Ege University School of Medicine, Izmir, Turkey

    Background/Purpose Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disorder characterized by recurrent episodes of fever accompanied by sterile peritonitis. The most devastating complication of…
  • Abstract Number: 1214 • 2014 ACR/ARHP Annual Meeting

    Oxidative Stress from Use of Allopurinol – Is There a Reason for Patients with Gout to Take Vitamin C?

    Lisa K. Stamp1, Peter T. Chapman2, John L. O'Donnell3, Irada Khalilova4, Rufus Turner4 and Anthony Kettle4, 1Department of Medicine, University of Otago, Christchurch, Christchurch, New Zealand, 2Rheumatology, Immunology & Allergy, Christchurch Hospital, Christchurch, New Zealand, 3Rheumatology Immunology & Allergy, Canterbury Health Laboratories, Christchurch, New Zealand, 4Pathology, University of Otago, Christchurch, Christchurch, New Zealand

    Background/Purpose During acute gout attacks neutrophils are activated and release a number of pro-inflammatory cytokines and enzymes. One of these enzymes is myeloperoxidase (MPO), which…
  • Abstract Number: 1212 • 2014 ACR/ARHP Annual Meeting

    Behcets Disease in Females Due to Mutation in NEMO, the NF-Kb Essential Modulator

    Alex Wessel1, Spiros Vonortas2, Jevgenia Zilberman-Rudenko3, Richard Siegel4 and Eric Hanson5, 15814 Beech Ave, NIH, Bethseda, MD, 2NIH, NIAMS, Bethesda, MD, 3Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 4NIAMS, NIH, Bethesda, MD, 5National Institute of Health, Bethesda, MD

    Background/Purpose: Behçet's disease (BD) is a chronic multi-system inflammatory disorder associated clinically with oral and genital ulceration, uveitis, erythema nodosum, and other inflammatory disease. The…
  • Abstract Number: 1213 • 2014 ACR/ARHP Annual Meeting

    Absence of Hormone Responsive Estrogen Receptor Alpha Reduces the Activation of Plasmacytoid Dendritic Cells in Lupus Prone Mice

    Jennifer L. Scott1, Melissa A. Cunningham2, Osama S. Naga3, Jackie G. Eudaly3, Jena R. Wirth4 and Gary S. Gilkeson5, 1Microbiology and Immunology, Medical University of South Carolina, Charleston, SC, 2Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, 3Medicine/Rheumatology, Medical University of South Carolina, Charleston, SC, 4Med/Rheumatology, MUSC, Charleston, SC, 5Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, SC

    Background/Purpose Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that affects women at a 9 to 1 ratio compared to men. To further understand…
  • Abstract Number: 1211 • 2014 ACR/ARHP Annual Meeting

    Gene Expression Profile in Muscle Tissue before and after Immunosuppressive Treatment in Patients with Myositis

    Joan Raouf1, Ingela M. Loell2, Yi-Wen Chen3, Rongye Shi4, Inger Nennesmo5, Helene Alexanderson6, Maryam Dastmalchi2, Marina Korotkova7, Kanneboyina Nagaraju8,9 and Ingrid E. Lundberg10,11, 1Department of Medicine, Rheumatology Unit, Karolinska University Hospital in Solna, Karolinska Institutet, Stockholm, Sweden, Stockholm, Sweden, 2Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Karolinska Institutet, Stockholm, Sweden, 3Research Center for Genetic Medicine, Children’s National Medical Center, Washington DC, USA., Washington DC, DC, 4Research Center for Genetic Medicine, Childrens National Medical Center, Research Center for Genetic Medicine, Washington DC, USA, Washington DC, WA, 5Institution for Laboratory Medicine (LABMED), Karolinska Universitetssjukhuset Huddinge, Stockholm, Sweden, Stockholm, Sweden, 6Karolinska Institutet, Department of medicine, Rheumatology Unit, Karolinska Universitetssjukhuset Solna, Stockholm, Sweden, Stockholm, Sweden, 7Department of Medicine, Rheumatology Unit, Department of Medicine, Karolinska University Hospital in Solna, Karolinska Institutet, Stockholm, Sweden, Stockholm, Sweden, 8Genetic Medicine, Children's National Medical Center, Washinton D C, DC, 9Research Center for Genetic Medicine, Children’s National Medical Center, Washington DC, DC, 10Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden, 11Rheumatology Unit, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden

    Background/Purpose Autoimmune muscle diseases such as polymyositis (PM) and dermatomyositis (DM) are characterized by infiltration of inflammatory cells, production of cytokines and chemokines, as well…
  • Abstract Number: 1210 • 2014 ACR/ARHP Annual Meeting

    The Role of the Transcription Factor cAMP Responsive Element Binding Protein 1 in Lipopolysaccharide-Induced Tolerance

    Kerstin Klein1, Renate E. Gay2, Christoph Kolling3, Lih-Ling Lin4, Steffen Gay1 and Caroline Ospelt1, 1Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Center of Experimental Rheumatology, Zurich University Hospital, Zurich, Switzerland, 3Schulthess Clinic, Zurich, Switzerland, 4Inflammation and Remodeling Research Unit, Pfizer, Cambridge, MA

    Background/Purpose In macrophages, repeated stimulation of Toll-like receptor (TLR) 4 leads to adaptation of signaling pathways and epigenetic modifications resulting in a tolerant state of…
  • Abstract Number: 1209 • 2014 ACR/ARHP Annual Meeting

    Anti-Scavenger Receptor Autoantibodies Disrupted Marginal Zone Macrophage Integrity Via Bruton’s Tyrosine Kinase

    Hao Li1,2, Qi Wu1, PingAr Yang1, Zheng Wang3, Jun Li1, Bao Luo1, Jeffrey C. Edberg1, Hui-Chen Hsu1, John D. Mountz1,4 and Robert P. Kimberly on behalf of PROFILE investigators5, 1Division of Clinical Immunology and Rheumatology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, 2Department of Medicine, Clinical Immunology & Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 3Med - Pulmonary/Allergy/Critical Care, University of Alabama at Birmingham, Birmingham, AL, 4Birmingham VA Medical Center, Birmingham, AL, 5Clinical Immun & Rheumatology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose Ibrutinib, a Btk kinase activity inhibitor, is a novel inhibitor under development for autoimmune disease therapy.  We have shown that Btk was significantly upregulated…
  • Abstract Number: 1208 • 2014 ACR/ARHP Annual Meeting

    Cholesterol Loading Induces Neutrophil Extracellular Traps, and Atorvastatin Attenuates This Effect

    Ming-Lin Liu1,2, Muhammad Bashir1,3, Kevin Williams4 and Victoria Werth5,6, 1Department of Dermatology, University of Pennsylvania, Philadelphia, PA, 2Department of Dermatology,, Philadelphia V.A. Hospital, Philadelphia, PA, 3Department of Dermatology, Philadelphia V.A. Hospital, Philadelphia, PA, 4Department of Medicine, Temple University School of Medicine, Philadelphia, PA, 5Dermatology, University of Pennsylvania, Philadelphia, PA, 6Dermatology, Philadelphia V.A. Hospital, Philadelphia, PA

    Background/Purpose Neutrophils are the most common white blood cell, but their role in autoimmune and cardiovascular diseases has been underestimated. As part of host defense,…
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